Kader Khan, who was known for his impeccable comic timing in films, died on the 31st Dec 2018 at the age of 81 in Canada after a prolonged illness. He was in the hospital for the last 16 – 17 weeks and was experiencing Progressive supranuclear palsy (PSP)

 

What is Progressive supranuclear palsy?

Progressive supranuclear palsy (PSP) is a disorder that has similar symptoms to Parkinson's disease (including unsteady gait, stiff movements, and mild dementia). Symptoms usually begin between the age of 50 and 60 but the range of the early forties to the late eighties is more common in men than in women. Because its early symptoms may look similar to those of Parkinson's disease or Alzheimer's disease, and sometimes it gets misdiagnosed with one of these two diseases.

PSP is a disorder caused by damage to certain nerve cells in the brain that outcomes in dynamic absence of coordination, the solidness of the neck and trunk, challenges with eye development, moderate developments, cognitive dysfunction, and difficulty walking that can result in falls.

The exact cause is unknown, but the disease gets worse over time. It involves damage to multiple cells of the brain, with loss of the myelin sheath (the covering of the nerve cell that speeds nerve impulse conduction) in some nerves and may lead to the destruction of the entire nerve in surrounding. The symptoms of PSP are caused by a gradual deterioration of brain cells in a few specific areas in the brain, mainly in the region called the brain stem. One of these areas, the substantia nigra, is also affected in Parkinson's disease, and damage to this region of the brain accounts in part for the motor symptoms that PSP and Parkinson's have in common.

People with this condition have deposits in brain tissues that resemble the deposits found in Alzheimer's disease. There is atrophy (loss of tissue) in most areas of the brain.

 

Symptoms may include:

• Repeated falls

• Loss of coordination, unsteady gait (walking pattern)

• Stiffness and rigid movement in the neck, trunk, arms, legs

• Difficulty in swallowing

• Pain or difficulty with bending the neck up or down

• Slow or stiff movements

• Vision difficulty

• Inability to look up or down without bending the neck

• Changes in facial expression

• Jaw or face jerks or spasms

• Pupils of the eyes different size

• Uncontrollable eye movements

• Generalized slowness of all movement with strength relatively preserved

• Speech difficulties

• Poor enunciation

• Low voice volume

• Mild dementia

• Forgetfulness

• Slowed thought processes

 

A neurological examination may demonstrate Parkinsonian developments with ordinary stiffness and absence of coordination. Eye developments are constrained especially vertical developments. Be that as it may, vision, hearing, sensation, and voluntary control of movement remain intact. Tests might be done to preclude different sicknesses. An MRI may indicate contracting of the brainstem.

 

How is PSP different from Parkinson's disease?

Stiffness, movement difficulties and clumsiness all these are caused by both PSP and Parkinson's disease, but PSP is more rapidly progressive as compared to Parkinson’s disease. People with PSP usually stand exceptionally straight or occasionally even tilt their heads backward (and tend to fall backwards). This is termed “axial rigidity.” Those with Parkinson's disease usually bend forward. Problems with speech and swallowing are much more common and severe in PSP than in Parkinson's disease and tend to show up earlier in the course of the disease. Eye movements are abnormal in PSP but close to normal in Parkinson's disease. Both diseases share other features: onset in late middle age, bradykinesia (slow movement), and rigidity of muscles. Tremor, very common in individuals with Parkinson's disease, is rare in PSP. Although individuals with Parkinson's disease markedly benefit from the drug levodopa, people with PSP respond minimally and only briefly to this drug. Also, people with PSP show accumulation of the protein tau in affected brain cells, while people with Parkinson’s disease show accumulation of a different protein, called alpha-synuclein.

 

How is PSP diagnosed?

No specific laboratory tests or imaging approaches currently exist to definitively diagnose PSP.  The disease is often difficult to diagnose because its symptoms can be very much like those of other movement disorders, and because some of the most characteristic symptoms may develop late or not at all. Initial complaints in PSP are typically vague and fall into these categories: 1) symptoms of disequilibrium, such as unsteady walking or abrupt and unexplained falls without loss of consciousness; 2) visual complaints, including blurred vision, difficulties in looking up or down, double vision, light sensitivity, burning eyes, or other eye trouble; 3) slurred speech; and 4) various mental complaints such as slowness of thought, impaired memory, personality changes, and changes in mood. An initial diagnosis is based on a person’s medical history and a physical and neurological exam. Diagnostic scans such as magnetic resonance imaging may show shrinkage at the top of the brain stem. Other imaging tests can look at brain activity in known areas of degeneration.

PSP is often misdiagnosed because it is relatively rare and some of its symptoms are very much like those of Parkinson's disease. Memory problems and personality changes may also lead a physician to mistake PSP for depression, or even attribute symptoms to some form of dementia. The key to diagnosing PSP is identifying early gait instability and difficulty moving the eyes, speech and swallowing abnormalities, as well as ruling out other similar disorders, some of which are treatable.

 

TREATMENT

There are very fewer medicines and drugs that are effective on the symptoms of PSP. Drugs like dopa, amantadine, amitriptyline, desipramine, and yohimbine can be used but unfortunately, none of these drugs provides dramatic or long-lasting improvements, and each can cause serious side effects. Botulinum toxin can be used to treat eyelid spasms and other types of dystonia that sometimes occur in PSP. In order to prevent drying of the eyes, artificial tears are helpful when blinking is decreased. 

Take help from a speech therapist who would teach safer swallowing techniques and advise on the need for a gastrostomy tube. The speech therapist can also offer the PSP patient alternative communication devices, such as pointing boards or computer-based systems. Books on tape provide an alternative when reading is no longer possible.

A weighted, delicate wheeled walker can help counteract falls. Caretakers can enhance the wellbeing of the individual with PSP by keeping pathways in the home clear of articles, for example, toys, floor carpets, or low furniture that are hard to see without looking down. An occupational specialist can prompt on these and other home adjustments to enhance wellbeing, solace, and ease of use of the home condition.

 

It is hard for us to lose such a brilliant actor. May his soul rest in peace.

 

https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Progressive-Supranuclear-Palsy-Fact-Sheet

https://brainfoundation.org.au/disorders/progressive-supranuclear-palsy

 

Written By - 

Shivani Sharma